|Year : 2015 | Volume
| Issue : 6 | Page : 792-796
A rare case of idiopathic orbital inflammatory syndrome
Ajit Kamalakar Joshi, Wilson Desai, Sakshi Patil, Asmita Devre
Department of Ophthalmology, Bharati Vidyapeeth Deemed University Medical College and Hospital, Sangli, Maharashtra, India
|Date of Web Publication||19-Nov-2015|
Ajit Kamalakar Joshi
Abhang, 248, 9/A, Anand Park, Road No. 2, Shinde Mala, Abhay Nagar, Sangli - 416 416, Maharashtra
Source of Support: None, Conflict of Interest: None
A 65-year-old woman presented with swelling and pain at the superomedial aspect of the right eye that was associated with proptosis, ptosis and restriction of eye movements superiorly and medially since 1 month. Considering the age and clinical presentation of the patient, a differential diagnosis of orbital cellulitis, thyroid orbitopathy, ocular myasthenia, orbital neoplasm, lymphoproliferative disorder and idiopathic orbital inflammatory syndrome (IOIS) was considered. Relevant laboratory investigations were performed, which were normal. Computed tomography, magnetic resonance imaging and biopsy were suggestive of IOIS. The patient was started on oral steroids and had a favorable outcome.
Keywords: Autoimmune, biopsy, diagnosis, etiology, idiopathic orbital inflammatory syndrome (IOIS), pseudotumor, treatment
|How to cite this article:|
Joshi AK, Desai W, Patil S, Devre A. A rare case of idiopathic orbital inflammatory syndrome. Med J DY Patil Univ 2015;8:792-6
| Introduction|| |
Idiopathic orbital inflammatory syndrome (IOIS), also known as orbital pseudotumor, is a non-infectious inflammation of the orbital soft tissues for which no cause is found after local and systemic evaluation.  Evaluation and management of patients with IOIS is challenging and the importance of its inclusion in the differential diagnosis of orbital disorders is necessary.  The aim of this study was to characterize the clinical features, histopathology, radiological evaluation and treatment outcomes in a rare case with biopsy-proven IOIS who had a favorable outcome with corticosteroid treatment.
| Case Report|| |
A 65-year-old woman presented with complaints of swelling and pain at the superomedial aspect of the right eye since 1 month. The swelling was associated with proptosis, lid edema and restriction of movements superiorly and medially since 1 month. There was no history of fluctuation of symptoms or diminution of vision. There was no history of fever, rash, joint pain or any other systemic symptoms. There was no history of preceding infection or trauma to the eye. There was no history of weakness in the limbs. There was no history of medications such as bisphosphonates, lithium and chemotherapeutics. There was no family history of similar complaints.
General examination was normal. On neurological examination, the cranial nerves were normal except extraocular movements. The extraocular movements were restricted superiorly and medially in the right eye. There was severe ptosis in the right eye. There was proptosis of 2.5 mm measured by the Hertel Exophthalmometer. There was conjunctival congestion and chemosis and the eyeball was deviated downwards and outwards laterally. Visual acuity, pupillary reaction and fundoscopy were normal. There was no audible bruit on auscultation over the right eye. The intraocular tension in both eyes was normal. The remainder of the neurological examination was normal [Figure 1]a-c.
|Figure 1: (a) Photograph of the patient showing complete ptosis. (b) Photograph of the patient showing conjunctival congestion and chemosis. (c) Photograph of the patient showing restriction of eye movements superiorly and medially|
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A differential diagnosis of orbital cellulitis, ocular myasthenia, orbital neoplasm and lymphoproliferative infiltration was considered. Total leukocyte count was normal with eosinophilia. The erythrocyte sedimentation rate was elevated. Liver, renal function and thyroid functions were normal. Antithyroglobulin (ATA) and antithyroid peroxidase antibodies (ATPAs) were negative. Antinuclear antibody was negative. Acetylcholine receptor antibodies and repetitive nerve conduction studies were negative.
Computed tomography (CT) with contrast showed evidence of a hypodense, peripherally enhancing lesion in the right orbit of size 30 mm × 16 mm × 29 mm in the extraconal compartment at the superomedial aspect, with normal orbital walls, cavernous sinus and brain [Figure 2].
|Figure 2: Pre-treatment computed tomography scan of the orbit with contrast showing a hypodense extraconal enhancing lesion|
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Magnetic resonance imaging (MRI) of the brain and orbit (plain) showed a multilocular heterogeneous T2 hyperintense lesion measuring approximately 30 mm × 16 mm × 23 mm in the right orbit, superomedial to the right eye globe. Infiltration of the orbital fat by the lesion in the superior quadrants of the right eye was seen [Figure 3].
|Figure 3: Pre-treatment magnetic resonance imaging of the brain and orbit showing T2 hyperintense lesion superomedial to the right eyeball|
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Histopathological examination of biopsy was suggestive of non-specific inflammation [Figure 4].
|Figure 4: Photograph of histopathological slide showing fibroadipose and fibrocollagenous tissue infiltrate by mononuclear cells|
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Considering the biopsy report, radiological features and negative blood reports, the patient was diagnosed as IOIS.
She was started on oral prednisolone (1 mg/kg/day) for 15 days followed by tapering doses over 10-12 weeks. The patient started showing response after 48 h and, at the end of 2 months, there was partial relief with improvement in ptosis and eye movements [Figure 5]a and b. A repeat computed tomography (CT) scan after 2 months showed reduction in the lesion and reduced infiltration of the fat [Figure 6]. The patient is on a maintenance dose of steroids and is kept under observation with monthly follow-up to rule out local recurrence or disease progression.
|Figure 5: (a and b) Post-treatment photographs of the patient showing improvement|
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|Figure 6: Post-treatment photograph of computed tomography scan of the orbit of the patient showing a resolving lesion|
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| Discussion|| |
IOIS is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorders.  The reported incidence of IOIS is between 4.7% and 6.3%. It represents approximately 5-8% of all orbital masses.  Disease usually occurs in adults, but may also affect children.  Pediatric IOIS encompasses about 6-16% in children, with a higher incidence of bilateral orbital involvement without evidence of underlying systemic disease.  Peak incidence appears to be predominantly in the adult population, typically in the middle-aged persons, and there is no sex predilection.  The disease has been reported in all ethnic groups around the globe.  In adults, IOIS tends to be unilateral.  Approximately 25% of cases present with bilateral disease, concurrently in 8% or subsequently in 14%.  The clinical course of IOIS ranges from mild and self-limiting to devastating orbital sclerosis with blindness. Disease relapse is common.  Forty percent of biopsy-proven IOIS cases can relapse.
The disease is also associated with lower socioeconomic status, high body mass index, younger age at first child birth in women and also use of oral bisphosphonate, lithium and chemotherapies.  Co-occurrence of certain rheumatological disorders such as Wegener's granulomatosis, sarcoidosis, giant cell arteritis, systemic lupus erythematosus, dermatomyositis and rheumatoid arthritis indicate that IOIS is an autoimmune disease. 
The clinical course of the disorder may be acute, subacute or chronic.  The lesion is most commonly restricted to the orbit; however, extension into adjacent retro - orbital structures is known. 
Pathogenesis and etiology of IOIS are currently unknown. Infectious processes such as upper respiratory infections and viral illness can be temporarily linked to the onset of IOIS.  Retroperitoneal fibrosis is a systemic disease entity that is similar in many respects to IOIS.  Evidence is emerging linking a subtype of IOIS with recently discovered clinical spectrum of IgG4-related systemic diseases.  Simultaneous occurrence of paranasal sinusitis and IOIS disease has been reported in the literature. 
Different anatomical subtypes of IOIS and their clinical presentations, with radiological features, are shown in [Table 1].
|Table 1: Different anatomical subtypes, their clinical presentation with radiological features|
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The uncommon presentations of IOIS include pediatric IOIS, optic nerve IOIS, scleritis, exudative retinal detachment, etc.
No single laboratory test is available to diagnose IOIS, but laboratory testing is performed to exclude other more specific diagnoses. The following tests are advised: Complete blood count, electrolytes, thyroid function tests, sedimentation rate, antinuclear antibodies, antineutrophil cytoplasmic antibodies, angiotensin-converting enzyme level, rapid plasma regain test, rheumatoid factor, etc.
Biopsy is not warranted in all cases and should be reserved for those with an atypical course.  or cases suspicious for an orbital malignancy or when a poor or equivocal response to corticosteroids is seen. 
Typical histopathology is characterized by a pleomorphic cellular infiltrate consisting of lymphocytes, plasma cells, macrophages, polymorphonuclear cells and eosinophils with a variable degree of reactive fibrosis. The other various histological findings are shown in [Table 2]. 
There exists controversy as whether sclerosing orbital inflammation belongs to true pseudotumor or is a separate clinicopathological entity. They have the tendency to involve the posterior orbit mainly. They require early and aggressive multiagent immunosuppressive therapy. Because of their aggressive nature, sometimes, radical surgery to debulk the tumor mass or orbital exenteration may be required.
IOIS remains a diagnosis of exclusion, after other identifiable local or systemic causes have been eliminated. A stepwise algorithm is presented to provide a more systematic approach to the diagnosis and treatment of this disease [Table 3].
Differential diagnoses of IOIS are as below [Table 4].
Only a minority of patients do well without treatment.  Corticosteroids are the cornerstone of therapy in acute cases. Around 37% of patients treated with steroids showed failure to resolve. In these situations, methotrexate, cyclophosphamides and other antineoplastic agents can be used. Radiotherapy is another modality of treatment. An alternative mode of treatment in recurrent and recalcitrant cases is with infliximab (TNF-alfa blocker).  In localized cases, surgery may be used. 
Treatment results in complete symptom relief in 63% of patients. However, 35% of patients experience only partial relief and are left with persistent motility dysfunction, pain or visual loss. In 2% of patients, treatment is ineffective. Of the patients who experience relief, one-third have recurrence of disease at the same or a different location. 
| Conclusion|| |
Understanding of the clinical features of patients with IOIS, differentiating it from other orbital processes by use of imaging techniques and biopsy and timely implementation of available treatment strategies may help prevent visual loss and associated morbidity from this condition.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
[Table 1], [Table 2], [Table 3], [Table 4]